Ectopic pituitary adenomas: common presentations of a rare entity

Agely, Ali; Okromelidze, Lela; Vilanilam, George K.; Chaichana, Kaisorn L.; Middlebrooks, Erik H.; Gupta, Vivek

doi:10.1007/s11102-019-00954-y

Cited by 22 publications

(34 citation statements)

References 22 publications

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“…Approximately 60% of reported ectopic pituitary adenomas are seen in the sphenoid sinus and suprasellar region, and 30% can be found in the clivus, nasal cavity, cavernous sinus, parasellar region, and sphenoid wing [ 19 ]. Rare sites beyond the migrational tract have also been described, including those in the petrosal temporal bone [ 20 ], superior orbital fissure [ 21 ], third ventricle [ 22 ], and temporal lobe [ 23 ]. These tumors typically respond to dopamine agonist to the same extent as eutopic prolactinomas.…”

Section: Discussionmentioning

confidence: 99%

“…The Medical Subject Headings (MeSH) search terms included ectopic, or paraneoplastic, and hyperprolactinaemia, or hyperprolactinemia and the search strategy for MEDLINE was constructed as follows: ((((ectopic) OR paraneoplastic) AND hyperprolactinaemia) OR hyperprolactinemia). Our systematic search revealed a total of 132 reports in the literature, most of which of cranial origin, including ectopic pituitary adenomas [ 9 – 11 ]. Our search identified six other reports of hyperprolactiaemia to a uterine neoplasm with only one case reporting this being secondary to a UTROSCT.…”

Section: Methodsmentioning

confidence: 99%

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Ectopic hyperprolactinaemia due to a malignant uterine tumor resembling ovarian sex cord tumors (UTROCST)

et al. 2020

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Purpose Moderate hyperprolactinaemia (2–5 times upper limit of normal) occurring in a patient with a normal pituitary MRI is generally considered to be due to a lesion below the level of detection of the MRI scanner assuming macroprolactin and stress have been excluded. Most patients with mild-to-moderate hyperprolactinaemia and a normal MRI respond to dopamine agonist therapy. We present the rare case of a patient who had prolactin elevation typical of a prolactin-secreting pituitary macroadenoma,with a normal cranial MRI, and in whom the prolactin rose further with dopamine agonist treatment. Subsequent investigations revealed ectopic hyperprolactinaemia to a uterine tumor resembling ovarian sex cord tumor (UTROSCT) which resolved following tumor resection. Although mostly considered to be benign, the UTROSCT recurred with recurrent hyperprolactinaemia and intraabdominal metastases. Methods We have systematically and critically reviewed existing literature relating to ectopic hyperprolactinaemia in general and UTROCST specifically. Results Fewer than 80 cases of UTROSCTs have been reported globally of which about 23% have shown malignant behaviour. There are fewer than 10 cases of paraneoplastic hyperprolactinaemia originating from uterine neoplasms including one other case of ectopic hyperprolactinaemia to a UTROSCT. Conclusions Our case demonstrates the importance of screening for extracranial hyperprolactinaemia in the context of: (1) substantially raised prolactin (10× ULN) and (2) normal cranial MRI assuming macroprolactin has been excluded. The majority of extracranial ectopic prolactin-secreting tumors occur in the reproductive organs.

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Section: Discussionmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Ectopic hyperprolactinaemia due to a malignant uterine tumor resembling ovarian sex cord tumors (UTROCST)

et al. 2020

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“…In this light, some authors suggest to routinely perform hormone evaluation in the presence of a parasellar lesion, since this may facilitate an early diagnosis and potentially avert an unnecessary biopsy and/or surgical intervention. Indeed, in recent years, some cases of PRL-secreting EPTs have been successfully treated using medical therapy with dopamine-agonists [50], and both GH-and TSH-secreting ETPs are supposed to benefit from treatment with somatostatin receptor ligands [57][58][59].…”

Section: Doi: 101159/000506905mentioning

confidence: 99%

“…The origin of EPTs has not been demonstrated yet. The currently accepted hypothesis suggests that these tumors originate from the proliferation of pituitary cells located along the embryological path of the pituitary development, during Rathke’s pouch migration in embryogenesis [50]. The most reported locations for parasellar EPTs are sphenoid sinus, suprasellar cistern, clivus, nasopharynx, and CS [51].…”

Section: Parasellar Lesions: Who Are the Players?mentioning

confidence: 99%

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Diagnosis and Treatment of Parasellar Lesions

et al. 2020

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The parasellar region, located around the sella turcica, is an anatomically complex area representing a crossroads for important adjacent structures. Several lesions, including tumoral, inflammatory vascular, and infectious diseases may affect this area. Although invasive pituitary tumors are the most common neoplasms encountered within the parasellar region, other tumoral (and cystic) lesions can also be detected. Craniopharyngiomas, meningiomas, as well as Rathke's cleft cysts, chordomas, and ectopic pituitary tumors can pri

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Clival ectopic pituitary prolactinoma was successfully managed by transsphenoidal surgery: A rare case report

Mansour,

Khozamah,

Naksho

et al. 2023

Clinical Case Reports

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Key Clinical MessageEctopic pituitary adenoma is a rare neoplasm located in the clivus and could mimic other clival tumors. Diagnosis and treatment could be challenging. It should be considered in the differential diagnosis of clival tumors.AbstractEctopic pituitary adenomas (EPAs) are isolated adenomas that can be located in variable locations outside the sella turcica and have a normal‐appearing pituitary gland. These tumors are rare and are thought to often arise from embryological remnants along the route of Rathke's pouch migration. EPAs are associated with a wide range of clinical manifestations depending on hormonal activity and involvement of adjacent structures, which can represent a challenge in making the diagnosis and deciding on the most appropriate management. In this case study, we report a 47‐year‐old male who presented with visual disturbances, a headache, and generalized weakness. Magnetic resonance imaging showed a 2 cm mass located in the clivus invading the sphenoid sinus with an intact pituitary gland. The patient underwent endoscopic transsphenoidal surgery to eradicate the mass while maintaining the integrity of the pituitary gland, which was successful and uneventful. Pathological studies were consistent with prolactinoma, with no cytological malignant features. Post‐surgery, symptoms notably improved, and serum prolactin levels significantly dropped, The patient's condition was satisfactory on follow‐up with no long‐term complications reported. This paper contributes to the existing literature by sharing the clinical management of a challenging and uncommon case.

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Ectopic pituitary adenomas: common presentations of a rare entity

Cited by 22 publications

References 22 publications

Ectopic hyperprolactinaemia due to a malignant uterine tumor resembling ovarian sex cord tumors (UTROCST)

Ectopic hyperprolactinaemia due to a malignant uterine tumor resembling ovarian sex cord tumors (UTROCST)

Diagnosis and Treatment of Parasellar Lesions

Clival ectopic pituitary prolactinoma was successfully managed by transsphenoidal surgery: A rare case report

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